The prognosis of subacute sclerosing panencephalitis in adults. Report of 8 cases and review of the literature.

In this article, 8 cases of subacute sclerosing panencephalitis (SSPE) are presented with a review of the literature. The symptoms developed during pregnancy in 2 cases. All of the patients died within 1 year duration except one. According to the reviewed literature, the prognosis in the adult cases is worse than in children; the two sexes are equally affected in adult SSPE.     

Sporadic and familial myoclonic dystonia: Report of three cases and review of literature

Myoclonic dystonia refers to a clinical syndrome characterized by rapid jerky movements along with dystonic posturing of the limbs. Clinically, it is characterized by sudden, brief, electric shock-like movements, mostly involving the upper extremities, shoulders, neck and trunk. Characteristically, the movements wane with consumption of small dose of alcohol in about 50% of cases. Additionally, dystonic contractions are observed in most of the patients in the affected body parts and some patients may exhibit cervical dystonia or graphospasm as well. It may manifest as an autosomal dominant condition or sometimes, as a sporadic entity, though there are doubts whether these represent cases with reduced penetrance. The condition is usually treated with a combination of an anticholinergic agent like, benztropine, pimozide and tetrabenazine. We report one sporadic case and one familial case where the father and the son are affected. The cases were collected from the Movement Disorders Clinic of Bangur Institute of Neurosciences, Kolkata, West Bengal in a period of ten months. Myoclonic dystonia is a rare condition and to the best of our knowledge, this series is the first one reported from our country. Videos of the patients are also provided with the article.     

Paraneoplastic subacute cerebellar degeneration in Hodgkin's disease Report of three cases and review of the literature

12 cases of paraneoplastic cerebellar degeneration with Hodgkin's disease have so far been reported. Three additional patients (Stage II-IA / IIA / IIIB) are presented. Although CT and MR-imaging (MRI) revealed a marked cortical atrophy of the cerebellum, there was no evidence of direct brain involvement. After irradiation and chemotherapy all patients showed a complete remission. There was no evidence of primary disease after an observation period of 3/2/5 years. Cerebellar symptoms also showed a partial remission.

As compared to the literature, our treatment appears to be more favourable, probably due to the fact, that therapy was more aggressive than usual.     

Hemifacial spasm as clinical presentation of intracranial meningiomas. Report of three cases and review of the literature

Hemifacial spasm (HFS) is a clinical entity consisting of brief clonic jerking movements of the facial musculature, beginning in the orbicularis oculi with downward spreading to other facial muscles. Apart from vascular loop compression at the root exit zone of the facial nerve, other causes of HFS are rare. It is exceptional as a form of presentation of intracranial meningiomas We report three cases of patients with meningiomas who presented with HFS, either as an isolated sign or associated with symptoms of rise intracranial pressure or focal deficit. We review the literature and discuss the possible physiopathological mechanisms responsible for this association.     

Suprasellar haemangioblastoma. Report of two cases and review of the literature.

We report 2 patients with suprasellar haemangioblastoma (HBL). The first, a 54-year-old man, presented with headache and gradually worsening bilateral visual field defects that had progressed to complete blindness on the right side. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain demonstrated a suprasellar mass. The mass was removed through a pterional craniotomy. The postoperative course was uneventful. He had no stigmata of von Hippel-Lindau (VHL) disease. After 5 years follow-up, vision in the left eye is normal but the right eye remains blind and MRI reveals no recurrence. The second, a 38-year-old man presented with a 2-month history of severe headaches and complete visual loss in the left eye. He had had surgery for excision of a cerebellar HBL, 5 years prior to this presentation. CT and MRI revealed a residual mass in the posterior fossa and a new suprasellar mass. He underwent craniotomy and subtotal excision of the suprasellar tumour. The histological diagnosis was HBL in both patients. HBL in the sellar and suprasellar region are rare and may be sporadic or occur in association with VHL disease. The literature is reviewed and diagnosis and treatment options discussed.     

Gliomatosis cerebri. Report of four cases and review of the literature

Four cases of gliomatosis cerebri are reported that demonstrate the variability of the clinical course. A review of these and 32 cases from the literature revealed that the most striking finding was change in personality and mental status. Hemiparesis, ataxia, papilledema, headache, seizure, and brainstem signs were the next most common clinical findings. Laboratory and radiographic tests, including angiography and pneumoencephalography, often showed only minor and nonspecific changes in the face of profound mental deterioration. Increased intracranial pressure usually occurred late but was probably an important factor of the terminal course in most patients. The pathologic changes were typical, with diffuse infiltration of astrocytoma cells through brain stem, subcortical white matter, and, to a lesser extent, cerebral cortex.     

Levodopa and melanoma: three cases and review of literature.

Three patients are reported who developed a melanoma while taking levodopa for Parkinson's disease. There were two cutaneous melanomas and one metastatic melanoma with occult primary. The literature on the association of Parkinson's disease, levodopa therapy and melanoma is reviewed. The capacity of levodopa to induce melanomas and its alleged adverse effect on the clinical course of the disease are questioned.     

Lupus-related myelopathy: report of three cases and review of the literature.

Transverse myelopathy is an uncommon complication of systemic lupus erythematosus (SLE). Three patients with SLE are reported who developed transverse myelopathy, including the neuropathological findings in one patient on whom necropsy was performed. Paraparesis was present in all three cases, but definite sensory changes were present in only one patient. In two patients, the CSF findings were remarkable for elevated protein and depressed glucose concentrations. Microscopic examination of the brain demonstrated small, scattered foci of recent necrosis consistent with microinfarctions. Striking abnormalities were found in the spinal cord at all levels, including multiple foci of vacuolar spongy degeneration in the peripheral white matter, as well as ballooning of myelin sheaths, swollen axons, myelin pallor, and loss of glial nuclei. The pathological findings in previously reported cases of SLE-related transverse myelopathy are reviewed, and the possible pathogenesis of the findings in our case are discussed.     

Spectrum of clinicoradiological findings in spinal cord infarction: Report of three cases and review of the literature

Spinal cord infarction (SCI) often remains undiagnosed due to infrequent occurrence and lack of established diagnostic procedures. The unique pattern of blood supply explains the heterogeneity of clinical presentation. We present three cases of SCI to highlight the varied spectrum of clinicoradiological findings. The first patient had posterior spinal artery infarction, and spine imaging showed infarction of adjacent vertebral body, which is usually rare. The second patient had anterior spinal artery infarction and the cANCA titers were elevated. The third patient had a pure motor quadriparesis. Initial imaging did not show any cord infarction, but signal changes were noted on serial imaging. Fibrocartilagenous embolism (FCE) seems the most likely etiology in the first and third cases. A high index of clinical suspicion is necessary for prompt diagnosis. Sensitivity of the initial magnetic resonance imaging remains limited, necessitating serial follow-up scans. Infarction of the adjacent vertebral body is a useful confirmatory sign. Fat suppression images can delineate the marrow signal changes better. Elderly patients with vascular risk factors and degenerative discs need to avoid mechanical triggers that predispose to FCE. Younger patients with SCI will need evaluation for cardioembolic source and vasculitis.     

Permanent lithium-induced cerebellar toxicity: three cases and review of literature.

Lithium can have toxic effects on the central nervous system that are both acute and chronic. Uncommonly, a long-lasting cerebellar syndrome follows acute toxicity. The mechanism of cerebellar injury is not well understood. We present 3 cases of this syndrome, with video demonstrating the typical features of this syndrome.     

Manifestations of Neuro-Behcet's disease. Report of two cases and review of the literature

Behcet's disease (BD) is a rare condition, more commonly seen in Turkey, the Middle East, the Mediterranean, and the Far Eastern countries. It is a multifaced disease involving the central nervous system (CNS), skin, joints, intestines and blood vessels. CNS involvement occurs in 10-25% of the patients with BD. We report two cases with Neuro-Behcet's disease. Both patients were females and they were in their second decade of life (early forties) when they suffered from relapsing retinitis and uveitis which led to blindness, despite treatment with steroids. They presented with clinical signs of brainstem syndrome, meningoencephalitis and the initial radiological investigations of our patients revealed encephalitic lesions in the brainstem. In the first case, an intracerebral abscess developed 4 years later, and it was evacuated under stereotactic guidance. The most common neuropathologic findings in Neuro-Behcet's disease are focal necrotic lesions in the brain. However, the association of Behcet's disease (BD) with cerebral abscess is very rare. There were only three reported cases of such. Ten years after the initial diagnosis of BD, our second patient suffered from subarachnoid hemorrhage due to a ruptured aneurysm of the superior cerebellar artery, which was then resected successfully. Intracranial aneurysm in patients with Behcet's disease has previously been documented in eight patients. To our knowledge, this is the only case of Neuro-Behcet's disease associated with an aneurysm of the superior cerebellar artery.     

Stroke following chiropractic manipulation. Report of 3 cases and review of the literature

We present 3 cases of stroke due to arterial dissection following chiropractic manipulation: (1) a 31-year-old woman with left vertebral dissection developed a large cerebellar infarct, (2) a 64-year-old man developed a left parietal infarct due to left carotid dissection and (3) a 51-year-old man developed right Horner's syndrome, fluctuating dysarthria, left facial droop, and left arm weakness due to right carotid dissection. Imaging studies and the literature are reviewed.     

Papillary tumor of the pineal region. Report of two cases and literature review

Papillary tumor of the pineal region (PTPR) was introduced to the WHO classification in 2007. This rare tumor of little known natural history and unpredictable behavior was described in fewer than 100 cases. Its optimal treatment is not established yet. We report another two cases of PTPR in whom tumors were totally removed via supracerebellar infratentorial approach and both were treated with radiotherapy. In a 37-year-old man the operation was delayed 6 years after the first tumor diagnosis and subsequent shunt placement. He has no complaints 10 years after the onset of the disease. A 45-year-old woman has no complaints 24 months after surgery. Our experience and the data from literature indicate that a total tumor removal is the major prognostic factor.     

Radiation-induced tumours of meninges. Report on eight cases and review of the literature

Background and purposeDespite their rarity, post-radiation meningeal tumours seem to be a growing problem due to the increasing application of radiation therapy. The aim of the study was to ascertain the specific features of these tumours.Material and methodsAmong 433 intracranial meningeal tumours treated from 2000 to 2008, eight cases (2%) have been presumed to be associated with high-dose therapeutic radiation for previous neoplasm of the head (7) or neck (1). On average, tumours were diagnosed 24 years after irradiation. All patients had a solitary meningeal tumour, but two of them also developed other neoplasms in the irradiated area.ResultsAll tumours were microsurgically removed. The postoperative course was uncomplicated in two cases only. In the remaining 6 (75%), complications included liquorrhoea (2), brain oedema (1), venous thrombosis (1), bleeding into the tumour bed (1) and focal deficit due to manipulation (3). Most tumours (5) were WHO grade I meningiomas. These benign meningiomas exhibited some peculiar histological features, including focal increase of cellularity, focal enhancement of proliferation index, pleomorphism of nuclei, occasional mitotic figures and, in one case, evidence of brain invasion. One meningioma was assigned to WHO grade II, one to WHO grade III and one appeared to be meningeal fibrosarcoma. The event-free survival and overall survival rate at 4.4 years of follow-up were 63% and 75%, respectively.ConclusionsRadiation-induced tumours of the meninges show certain characteristic histopathological features, which may promote invasiveness of the tumour and higher risk of malignancy.     

Mucoceles of the sphenoidal sinus. Report of six cases and review of the literature

Six personal cases of mucocele of the sphenoidal sinus are reported, and 124 cases from the literature are reviewed. Sphenoidal sinus mucocele is a benign and rare lesion which has long been unrecognized. Clinical features include fronto-orbital pain, oculomotor palsies, loss of visual acuity, exophthalmos and anosmia. Our series is of particular interest since four of our six patients presented with endocrine disorders. Recent advances in neuroradiological methods should rapidly lead to the correct diagnosis. Treatment consists of aspiration and drainage of the mucocele via a trans-sphenoidal approach. The results are good, and ophthalmoplegia usually subsides. In our experience, endocrine disorders do not always respond to treatment. The prognosis of sphenoidal sinus mucocele depends on the preoperative duration of the loss of visual acuity.     

Paraganglioma of the cauda equina. Report of 2 cases and review of 59 cases from the literature

Paragangliomas of the cauda equina are not so rare as said in the literature. Two additional cases are presented with a global analysis of the 59 cases from the literature. The diagnosis of this pathology greatly benefit of the use of immunostainings as the cells are often neuron-specific enolase, neurofilament protein and somatostatin positive so that electron microscopy is thus no longer mandatory for establishing the diagnosis. In addition, we report the first magnetic resonance images of this tumor at this location.     

Aicardi syndrome. Report of 6 cases and a review of Japanese literature

This report presents 6 cases of Aicardi syndrome (infantile spasms, agenesis of the corpus callosum, chorioretinopathy), and a review of Japanese literature. Two patients developed Lennox-Gastaut syndrome following infantile spasms. Visual evoked potential was examined in every case with binocular and monocular flash stimulations. Normal response was obtained in 5 cases, and no response in 1. Auditory brainstem response was examined in 2 cases. Central conduction time was prolonged in 1 case.     

Autoimmune cerebellar hypermetabolism: Report of three cases and literature overview

We report three cases of vermian cerebellar hypermetabolism in patients with autoimmune encephalitis. One of our patients was positive for anti-Ma2 antibodies and one for anti-Zic4 antibodies while the remaining patient did not present any known antibodies. The seronegative patient deteriorated after immune checkpoint inhibitor treatment for a pulmonary adenocarcinoma and improved with immunosuppressive drugs, which is in favour of an underlying autoimmune mechanism. They all presented with subacute neurological symptoms. Brain magnetic resonance imaging was normal except in one patient, where hyperintensities were present on FLAIR sequence around the third ventricle and the cerebral aqueduct. ¹⁸F-FDG brain positron emission tomography with computed tomography (¹⁸F-FDG PET-CT) demonstrated an unusual vermian cerebellar hypermetabolism in the three cases. While cerebellar hypermetabolism on ¹⁸F-FDG PET-CT has been described in various neurological diseases, such vermian – and more broadly cerebellar – hypermetabolism was seldom described in previous studies on autoimmune encephalitis. When differential diagnoses have been ruled out, this pattern may be of interest for the positive diagnosis of autoimmune encephalitis in difficult diagnostic cases.